Calciphylaxis

Overview

Calciphylaxis is a rare and serious disease in which calcium accumulates in the small blood vessels of the fat and skin tissues. This condition causes blood clots, painful skin ulcers, and can lead to serious infections, potentially resulting in death. People with calciphylaxis usually have kidney failure and are either on dialysis or have undergone a kidney transplant. The condition can also occur in those without kidney disease. Symptoms include large purple net-like patterns on the skin, deep, very painful lumps that ulcerate and create open sores with black-brown crust that fails to heal, and infections from wounds that don't heal. Risk factors include being female, obesity, diabetes, abnormalities in blood-clotting factors, long-term dialysis or kidney transplantation, imbalances in calcium, phosphorus, and aluminum, certain medications, overproduction of parathyroid hormone, and uremia. Complications can include severe pain, large non-healing ulcers, blood infections, and death due to infection and multi-organ failure. Early detection and treatment are crucial for preventing serious infections.

Understanding the disease

Development

Calciphylaxis develops when there is an imbalance in the metabolism of calcium, leading to calcium deposition in the smallest parts of the arterioles. This causes blood clots to form, which can obstruct the flow of oxygen and nourishment to fat tissues and skin. The condition is primarily associated with end-stage kidney failure and is more common in individuals with risk factors such as being female, obesity, diabetes, abnormalities in blood-clotting factors, long-term dialysis, and sometimes kidney transplantation. Calciphylaxis can cause severe pain, large, deep, nonhealing ulcers, blood infections, and may lead to death, usually due to infection and multiorgan failure.

Assessment and Diagnosis

Diagnosis

To diagnose calciphylaxis, your doctor will review your medical history, assess your symptoms, and perform a physical exam. Tests that may be conducted to determine if you have calciphylaxis include a skin biopsy, blood tests to measure various substances in your blood, and imaging studies like X-rays to check for vascular calcifications.

Management and Treatment

Complications

Potential complications of calciphylaxis include severe pain, large, deep, nonhealing ulcers, blood infections, and death, usually due to infection and multiorgan failure. Calciphylaxis can lead to serious infections, and early detection and treatment is important to prevent these complications.

Treatment

Treatment for calciphylaxis typically involves multiple interventions, including:

1. Restoring oxygen and blood flow to the skin: Medication like anticoagulation medication, such as apixaban (Eliquis), may be prescribed to restore blood flow to affected tissues. Hyperbaric oxygen therapy can also help increase oxygen delivery to the affected parts of the body.
2. Decreasing calcium deposits: Your doctor may change your dialysis prescription, modify your current medications, and adjust your calcium or vitamin D supplements to eliminate potential triggers for calciphylaxis.
3. Taking medications: Medications like sodium thiosulfate can decrease calcium buildup in the arterioles. Cinacalcet (Sensipar) can help control parathyroid hormone (PTH), while other medications may be used to improve the balance of calcium and phosphorus.
4. Surgery: If necessary, surgery to remove an overactive parathyroid gland may be recommended.
5. Intensive wound treatment: Debridement or other methods may be used to remove damaged tissue, and antibiotic treatment may be prescribed to manage infection. It is essential to work closely with your doctor to determine the most appropriate treatment plan based on your specific condition and medical history.

Preparing for medical consultation

To prepare for an appointment for calciphylaxis, you should:

1. Ask your healthcare provider if there are any pre-appointment restrictions, such as dietary changes you need to make before the appointment.
2. Make a list of your symptoms, including those that may seem unrelated to calciphylaxis.
3. Write down key personal information, such as major stresses, recent life changes, and family medical history.
4. Create a list of all medications, vitamins, or supplements you're currently taking, including doses.
5. Consider bringing a family member or friend with you for support and to help remember important information.
6. Prepare a list of questions to ask your healthcare provider during the appointment. Remember to bring any previous scans, X-rays, or medical records relevant to your situation to the appointment. This will help your healthcare provider understand your condition better and make informed decisions about your treatment.